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  • A 55-year-old woman comes to the emergency department complaining of abdominal pain. She states that she has suffered intermittent nausea for 2 years. Then 3 months ago she began to notice right-sided abdominal pain. She denies vomiting, diarrhea, or constipation. She has not been to a doctor in 3 years since she moved from Peru. She has had no surgeries. She takes no medications. On physical examination, there is moderate right upper quadrant tenderness. An ultrasound shows a 5-cm cyst in the right upper lobe of the liver with an irregular, calcified wall.
Introduction
  • Clinical definition
    • liver cyst caused by Echinococcus tapeworm
  • Epidemiology
    • demographics
      • most common form is E. granulosus and E. multilocularis
      • usually acquired during childhood but do not manifest symptoms until adulthood
    • risk factors
      • geographic
        • South America, Middle East, sub-Saharan Africa, and western China
        • close contacts with dogs and sheep 
  • Pathogenesis
    • definitive host
      • usually dogs
    • intermediate hosts include
      • sheep, goats, camels, horses, cattle, and swine
    • transmission
      • adult tapeworm inhabits small intestine
      • produces eggs that are expelled in stool
      • eggs ingested by incidental host (fecal-oral transmission)
      • parasites hatch from eggs and penetrate intestinal mucosa to enter blood/lymphatic system
      • migrates to liver or other organs to form hydatid cysts
    • humans are incidental hosts
Presentation
  • Symptoms 
    • mostly asymptomatic
    • if liver involved
      • 2/3 of the time
      • nausea, vomiting, and right upper quadrant pain
    • if lung involved
      • 25% of the time
      • cough, chest pain, dyspnea, and hemoptysis
  • Physical exam
    • hepatosplenomegaly
Studies
  • Diagnostic testing
    • imaging
      • ultrasound
        • best initial test
          • inexpensive
          • 90-95% sensitive
        • single anechoic, smooth, and round cyst
        • may have septations
        • may have a thick or irregular wall
        • “eggshell” appearance if calcified
        • may have daughter cysts (peripherally based cyst within a cyst)
      • computed tomography or magnetic resonance imaging
        • 95-100% sensitivity
        • for greater anatomic detail to establish location and number of cysts, presence of ruptured or calcified cysts, and to guide management
        • better for evaluation of extrahepatic cysts
    • studies
      • complete blood count
        • mild eosinophilia
        • mild elevation in liver function labs
      • serologic and antigen assays
        • i.e., enzyme-linked immunosorbent assay (ELISA)
        • can use for primary diagnosis and follow-up after treatment
        • a negative serologic test does not rule out echinococcosis
      • cyst aspiration/biopsy
        • if serologic test indeterminate/negative
        • risk of anaphylaxis and secondary spread of infection
Differential
  • Simple cyst
    • distinguishing factors
      • thinner wall, no calcifications, no septations, and no daughter cysts
      • sterile fluid if aspirated
      • negative serology
        • no additional tests necessary if imaging findings are consistent with a simple cyst
  • Cystadenoma or cystadenocarcinoma
    • distinguishing factors
      • rapid growth
      • tumor cells on histopathology
        • biliary-type mucus-secreting cuboidal or columnar epithelium for cystadenoma
        • malignancy changes of inner epithelial lining for cystadenocarcinoma
      • may have elevated levels of carcinoembryonic antigen (CEA)
      • negative serology
Treatment
  • First-line
    • albendazole
      • indication
        • single cyst < 5 cm
      • mechanism of action
        • inhibits microtubule assembly
      • adverse effects
        • hepatotoxicity
        • cytopenia
        • alopecia
        • rash
  • Second-line
    • image-guided percutaneous drainage
      • indication
        • cysts 5-10 cm
        • must be done in combination with medical therapy
      • adverse effect
        • risk of seeding
        • risk of anaphylaxis
  • Third-line
    • resection
      • indication
        • cysts > 10 cm
        • complicated cysts
          • associated with rupture, infection, compression/mass effect, biliary fistulae, hemorrhage, multiple daughter cysts, or extrahepatic cysts
      • adverse effect
        • risk of seeding and anaphylaxis less than percutaneous drainage as attempts is to resect the whole cyst
  • Other treatments
    • Mebendazole and praziquantel are less effective
Complications
  • Mass effect
    • Budd-Chiari syndrome
    • portal hypertension
    • cholestasis
    • cirrhosis
  • Secondary bacterial infection
  • Cyst rupture
    • presentation
      • fever
      • acute hypersensitivity reaction (i.e., anaphylaxis)
      • obstructive jaundice
      • death
 

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Questions (2)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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